Objectives: To report our experience with sudden death in children with septo-optic dysplasia and to identity specific risk factors and suggest preventive measures to minimize mortality. Methods: Clinical data from 5 children with septo-optic dysplasia who died suddenly and unexpectedly were evaluated retrospectively. Results: All children had corticotropin deficiency, all had thermoregulatory disturbances, and 4 children had diabetes insipidus. In at least 4 children, clinical deterioration was caused by lever and dehydration from a presumed viral illness, which appeared to precipitate adrenal crisis. Conclusions: Children with septo-optic dysplasia and hypocortisolism are at risk for sudden death during febrile illness. Thermoregulatory disturbances and dehydration from diabetes insipidus may potentiate clinical deterioration. Prevention of sudden death in septo-optic dysplasia requires early recognition and treatment of these major risk factors.
|Original language||English (US)|
|Number of pages||5|
|Journal||Archives of ophthalmology|
|State||Published - 1997|
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